{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T04:09:44Z","timestamp":1695528584412},"reference-count":27,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,4,8]],"date-time":"2015-04-08T00:00:00Z","timestamp":1428451200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Respiratory J"],"published-print":{"date-parts":[[2016,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aims<\/jats:title><jats:p>Primary ciliary dyskinesia (<jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content>) is a rare, genetic disorder featured with dysfunctional motility of cilia. Clinical presentations of <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> include situs inversus, repeated respiratory tract infections, otitis media, sinusitis and infertility. This study aims to provide clinical strategies on diagnosis and treatment of <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical data of seven patients diagnosed as <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">Z<\/jats:styled-content>hongshan <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>ospital, <jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>udan <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>niversity were analyzed. We also extracted data from other cases in mainland <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hina from the <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hina <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>cademic <jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>ournals <jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>ull\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>ext <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>atabase by the end of 2012. A total of 127 cases of <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> were summarized and analyzed in this study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seven patients in <jats:styled-content style=\"fixed-case\">Z<\/jats:styled-content>hongshan <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>ospital, <jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>udan <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>niversity were finally confirmed to have <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content>. All of them had clinical history of recurrent respiratory infection. Imaging analysis showed varying degrees of bronchiectasis. Pulmonary function tests in four patients showed combined obstructive and restrictive patterns. Three cases were confirmed to have <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> by electron microscopy.<\/jats:p><jats:p>We then further extracted information from literature for those 127 <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> patients in mainland <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hina. All of them had chronic respiratory infection. Seven cases were complicated with recurrent otitis media and 15 cases were complicated with infertility. A total of 119 patients were diagnosed with situs inversus totalis (94%). Among those 127 patients, only 9 patients' diagnosis was confirmed by electron microscopy. Four patients received sinus resection, and one patient received pulmonary lobectomy due to recurrent hemoptysis. One patient died of massive hemoptysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Being a rare disease and easily confused with uncomplicated bronchiectasis, <jats:styled-content style=\"fixed-case\">PCD<\/jats:styled-content> diagnosis warrants particular attention. Recurrent respiratory infection, especially situs inversus could be the most important indicator of this disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/crj.12284","type":"journal-article","created":{"date-parts":[[2015,3,12]],"date-time":"2015-03-12T11:07:08Z","timestamp":1426158428000},"page":"765-771","source":"Crossref","is-referenced-by-count":6,"title":["Clinical analysis of patients with primary ciliary dyskinesia in mainland <scp>C<\/scp>hina"],"prefix":"10.1111","volume":"10","author":[{"given":"Yueqin","family":"Cao","sequence":"first","affiliation":[{"name":"Shanghai Respiratory Research Institute Department of Pulmonary Medicine Zhongshan Hospital Fudan University  Shanghai China"},{"name":"Department of Pulmonary Medicine The Forth People's Hospital of Taizhou  Taizhou Jiangsu Province China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Changzhou","family":"Shao","sequence":"additional","affiliation":[{"name":"Shanghai Respiratory Research Institute 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